In an effort to identify pathways for improvements in clinical monitoring and intervention, the current study investigated the role that parenting style plays in treatment adherence and responsibility for youth with CF. Participants (n = 50) completed questionnaires related to treatment adherence, treatment responsibility, and parenting style at baseline and at a two year follow up visit. Aspects of parenting style (e.g., warmth, autonomy support) were related to youth adherence and responsibility in cross-sectional and prospective analyses. These data suggest that aspects of parenting may be important targets of interventions to promote treatment adherence in youth with CF.